Small Bowel/Small Bowel-Liver/Multivisceral Transplantation
DESCRIPTION
Small bowel transplant is the transplantation of an intestinal allograft to an individual with irreversible intestinal failure to restore intestinal function. Intestinal failure is the inability of the small bowel to absorb adequate nutrition and fluids due to loss in length, function, and/or absorptive capacity of the small bowel, resulting in malabsorption, malnutrition, and dehydration. The most common cause of intestinal failure is short bowel syndrome (SBS) or short gut syndrome, a congenital disorder in which an infant's intestine is too short or underdeveloped to allow normal food digestion. Other causes may include abdominal trauma, Crohn's disease, thrombotic disorders and surgical adhesions.
Small bowel transplantation can be performed in one of three ways: alone, in combination with the liver, or multi-visceral (i.e., with one or more of the following: liver, pancreas, stomach, duodenum, intestine and colon).
Total parenteral nutrition (TPN) can produce long-term survival once small intestinal dysfunction makes oral nutrition ineffective. Complications resulting from TPN use may lead to serious morbidity and mortality.
POLICY
Small bowel transplantation for the treatment of irreversible intestinal failure is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Small bowel - liver transplantation for the treatment of irreversible intestinal failure is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Multivisceral transplantation for the treatment of irreversible intestinal failure is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Repeated labs and procedures are considered medically necessary to address changes in condition and for continued transplant listing.
Multiple labs and work-up procedures are considered not medically necessary for the sole purpose of repeat evaluation at multiple transplant centers.
MEDICAL APPROPRIATENESS
Small bowel transplantation is considered medically appropriate with ALL of the following:
ANY ONE of the following:
The individual is negative for human immunodeficiency virus (HIV)
No advancing HIV disease as indicated by ALL the following:
The CD4 count is greater than 200 cells per cubic millimeter for greater than 6 months
The HIV-1 RNA level is undetectable
An individual has been stable on anti-retrovial therapy greater than 3 months
No other complications from acquired immune deficiency syndrome (AIDS) (e.g. opportunistic infections, including aspergillus, tuberculosis, coccidiosis mycosis, resistant fungal infections Kaposi’s sarcoma or other neoplasm)
ANY ONE of the following:
Small bowel transplantation (cadaveric or living donor) for the treatment of irreversible intestinal failure with ALL of the following:
Clinical information submitted for determination of medical appropriateness criteria is dated within the last seven months
Total parenteral nutrition (TPN) has failed
ABSENCE of alcohol or drug use with ANY ONE of the following:
No history of alcohol or drug use
In individuals with a history of alcohol or drug use, ALL of the following:
Documented six month abstinence from drug or alcohol use
Ongoing participation in a formal treatment program
ANY ONE of the following:
Short gut syndrome with loss of over 70% of the native small bowel
Defective intestinal motility (e.g., hollow visceral myopathy, neuropathy, and / or total intestinal aganglionosis)
Impaired enterocyte absorptive capacity (e.g., microvillus inclusion disease, selective autoimmune enteropathy, radiation enteritis, extensive inflammatory bowel disease and / or massive intestinal polyposis)
Failure of transplanted graft of small bowel
ABSENCE of absolute contraindications, including ALL of the following:
Life expectancy of less than five years due to age-related debilitation and co-morbidities
Ability to ingest oral nutrition
Unresectable malignancy
Serious, uncontrolled psychiatric illness that would hinder compliance with any stage of the transplant process
Neurologic illness independent of the disease process being treated
Active substance abuse (alcohol, drugs, or other toxins)
Active and / or life-threatening infection
Severe body / organ system disease unrelated to transplanted organ
Compromised cardio-pulmonary function unrelated to transplanted organ
Inability or unwillingness of the individual or legal guardian to give signed consent and to comply with regular follow-up requirements
Small bowel - liver transplantation with ALL of the following:
Clinical information submitted for determination of medical appropriateness criteria is dated within the last 7 months
Total parenteral nutrition (TPN) has failed
ANY ONE of the following:
Irreversible failure of both liver and intestines
Liver failure associated with total thrombosis of the portomesenteric system
ABSENCE of alcohol or drug use with ANY ONE of the following:
No history of alcohol or drug use
In individuals with a history of alcohol or drug use, ALL of the following:
Documented six month abstinence from drug or alcohol use
Ongoing participation in a formal treatment program
ABSENCE of absolute contraindications, including ALL of the following:
Extrahepatic malignancy including cholangiocarcinoma with the past five years with the exception of basal cell and squamous cell carcinoma of skin
Hepatocellular carcinoma that has extended beyond the liver
Uncontrolled systemic sepsis
Active substance abuse (e.g., alcohol, drugs)
Irreversible advanced cardiac, pulmonary, renal, neurologic or other organ disease
Evidence of significant non-compliance
Active substance abuse (alcohol, drugs, or other toxins)
History of alcohol or drug use, must meet ALL of the following:
Documented six month abstinence from drug or alcohol use
Ongoing participation in a formal treatment program
Medical therapy has been optimal and no surgical procedure other than transplantation offers a realistic expectation of functional improvement and extension of life, in the presence of end-stage liver failure due to an irreversibly damaged liver
ANY ONE of the following:
Hepatocellular with ANY ONE of the following:
Cryptogenic cirrhosis
Chronic viral hepatitis
Autoimmune hepatitis
Alpha-1 antitrypsin deficiency
Protoporphyria.
Alcoholic cirrhosis including ALL of the following:
Confirmation of the abstinence of alcoholic for six months
Ongoing participation in a formal treatment program
Fulminant hepatic failure with ANY ONE of the following:
Viral hepatitis (if the etiology is thought to be related to IV drug use) with ALL the following:
Confirmation of the abstinence from IV drugs for six months
Ongoing participation in a formal treatment program
Drugs or toxins with ALL the following:
Confirmation of the abstinence from IV drugs for six months
Ongoing participation in a formal treatment program
Wilson’s disease
Cholestatic liver diseases with ANY ONE the following:
Biliary cirrhosis (primary or secondary)
Sclerosing cholangitis
Biliary atresia
Vascular disease (e.g., Budd-Chiari syndrome)
Primary hepatocellular carcinoma
Metabolic disorders with ANY ONE of the following:
Hemochromatosis
Glycogen storage disease
Familial hypercholesterolemia
Trauma and toxic reactions
Polycystic disease of the liver; with ANY ONE of the following:
Enlargement of liver impinging on respiratory function
Extremely painful enlargement of liver
Enlargement of liver significantly compressing and interfering with function of other abdominal organs
Familial amyloid polyneuropathy when the individual is a liver transplant candidate based on the morbidity of the polyneuropathy
Multivisceral transplantation with ALL of the following:
Clinical information submitted for determination of medical appropriateness criteria is dated within the last 7 months
Total parenteral nutrition (TPN) has failed
ANY ONE of the following:
Combined organ failure and/or premalignant conditions of the gastrointestinal tract, which includes three or more of the abdominal visceral organs including the small bowel
Extensive thrombosis of the splanchnic vascular system, massive gastrointestinal polyposis, and generalized hollow visceral myopathy or neuropathy
ABSENCE of alcohol or drug use with ANY ONE of the following:
No history of alcohol or drug use
In individuals with a history of alcohol or drug use, ALL of the following:
Documented six month abstinence from drug or alcohol use
Ongoing participation in a formal treatment program
ABSENCE of absolute contraindications, including ALL of the following:
Extrahepatic malignancy including cholangiocarcinoma with the past five years with the exception of basal cell and squamous cell carcinoma of skin
Hepatocellular carcinoma that has extended beyond the liver
Uncontrolled systemic sepsis
Active substance abuse (e.g., alcohol, drugs)
Irreversible advanced cardiac, pulmonary, renal, neurologic or other organ disease
Evidence of significant non-compliance
Active substance abuse (alcohol, drugs, or other toxins)
History of alcohol or drug use, must meet ALL of the following:
Documented six month abstinence from drug or alcohol use
Ongoing participation in a formal treatment program
Medical therapy has been optimal and no surgical procedure other than transplantation offers a realistic expectation of functional improvement and extension of life, in the presence of end-stage liver failure due to an irreversibly damaged liver
ANY ONE of the following:
Hepatocellular with ANY ONE of the following:
Cryptogenic cirrhosis
Chronic viral hepatitis
Autoimmune hepatitis
Alpha-1 antitrypsin deficiency
Protoporphyria
Alcoholic cirrhosis including ALL of the following:
Confirmation of the abstinence of alcoholic for six months
Ongoing participation in a formal treatment program
Fulminant hepatic failure with ANY ONE of the following:
Viral hepatitis (if the etiology is thought to be related to IV drug use) with ALL the following:
Confirmation of the abstinence from IV drugs for six months
Ongoing participation in a formal treatment program
Drugs or toxins with ALL the following:
Confirmation of the abstinence from IV drugs for six months
Ongoing participation in a formal treatment program
Wilson’s disease
Cholestatic liver diseases with ANY ONE the following:
Biliary cirrhosis (primary or secondary)
Sclerosing cholangitis
Biliary atresia
Vascular disease (e.g., Budd-Chiari syndrome)
Primary hepatocellular carcinoma
Metabolic disorders with ANY ONE of the following:
Hemochromatosis
Glycogen storage disease
Familial hypercholesterolemia
Trauma and toxic reactions
Polycystic disease of the liver; with ANY ONE of the following:
Enlargement of liver impinging on respiratory function
Extremely painful enlargement of liver
Enlargement of liver significantly compressing and interfering with function of other abdominal organs
Familial amyloid polyneuropathy when the individual is a liver transplant candidate based on the morbidity of the polyneuropathy
IMPORTANT REMINDER
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.
ADDITIONAL INFORMATION
Examples of the indications for failed total parenteral nutrition (TPN) can include any of the following: impending liver failure due to TPN-induced liver injury, limited central venous access, frequent central line infection and sepsis or frequent episodes of severe dehydration despite intravenous fluid supplementation with TPN.
The center responsible for the organ harvesting should comply with the U.S. Department of Health and Human Services Organ Procurement and Transplantation Network (OPTN) guidelines.
Small bowel transplants should be performed in a facility that is licensed, accredited, and approved by Medicare as a Transplant Center. The Medicare list is available at http://www.cms.gov/CertificationandComplianc/Downloads/ApprovedTransplantPrograms.pdf.
SOURCES
Abu-Elmagd, K. M., Costa, G., Bond, G. J., Soltvs, K., Sindhi, R., Wu, T., et al. (2009). Five hundred intestinal and multivisceral transplantations at a single center: Major advances with new challenges. Annals of Surgery, 250 (4), 567-581. (Level 1 Evidence - Independent study)
American Association for the Study of Liver Diseases. (2008, September). AASLD practice guidelines: Evaluation of the patient for liver transplantation. Retrieved February 18, 2011 from http://www.aasld.org/practiceguidelines/Documents/Bookmarked%20Practice%20Guidelines/Liver%20Transplant.pdf.
American Gastroenterological Association. (2003, April). American Gastroenterological Association medical position statement: Short bowel syndrome and intestinal transplantation. Retrieved November 19, 2010 from http://download.journals.elsevierhealth.com/pdfs/journals/0016-5085/PIIS0016508503000520.pdf.
Bhagani, S., Sweny, P., Brook, G., & British HIV Association. (2006). Guidelines for kidney transplantation in patients with HIV disease. HIV Medicine, 7 (3), 133-139.
BlueCross BlueShield Association. Medical Policy Reference Manual. (10:2010). Isolated small bowel transplant (7.03.04). Retrieved November 19, 2010 from BlueWeb. (17 articles and/or guidelines reviewed)
BlueCross BlueShield Association. Medical Policy Reference Manual. (6:2011). Small bowel/liver and multivisceral transplant (7.03.05). Retrieved August 24, 2011 from BlueWeb. (11 articles and/or guidelines reviewed)
Complete Guide to Medicare Coverage Issues [Computer software]. (2011, April). Adult liver transplantation. (NCD 260.1, p. 2-209, 2-210). Ingenix.
Complete Guide to Medicare Coverage Issues [Computer software]. (2011, April). Pediatric liver transplantation. (NCD 260.2, p. 2-210). Ingenix.
Complete Guide to Medicare Coverage Issues [Computer software]. (2011, April). Intestinal and multi-visceral transplantation (NCD 260.5, p. 2-212). Ingenix.
Complete Guide to Medicare Coverage Issues. (2008, October). Medicare approved adult and pediatric intestinal transplant centers. Retrieved February 18, 2011 from http://www.uptodate.com/contents/image?imageKey=PI/18468.
Lao, O. B., Healey, P. J., Perkins, J. D., Reyes, J. D., & Goldin, A. B. (2010). Outcomes in children with intestinal failure following listing for intestinal transplant. Journal of Pediatric Surgery, 45 (1), 100-107.
Steinman, T. I., Becker, B. N., Frost, A. E., Olthoff, K. M., Smart, F. W., Suki, W. N., et al. (2001). Guidelines for the referral and management of patients eligible for solid organ transplantation. Transplantation, 71 (9), 1189-1204.
Townsend, C. M., Jr., Beauchamp, R. D., Evers, B. M., & Mattox, K. L. (Eds.). (2008). Sabiston Textbook of Surgery (18th ed., Chapter 28). Philadelphia: W. B. Saunders Company.
U.S. Department of Health and Human Services. Organ Procurement and Transplantation Network (OPTN). (2010, November). Identification of transmissible diseases in organ recipients. Retrieved August 24, 2011 from http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_16.pdf.
U.S. Department of Health and Human Services. Organ Procurement and Transplantation Network (OPTN). (2010, November). Allocation of livers. Retrieved November 19, 2010 from http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_8.pdf.
U.S. Department of Health and Human Services. Organ Procurement and Transplantation Network (OPTN). (2010, November). Allocation system for organs not specifically addressed. Retrieved November 19, 2010 from http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_11.pdf.
U.S. Department of Health and Human Services. Organ Procurement and Transplantation Network (OPTN). (2008, June). Intestinal organ allocation. Retrieved November 19, 2010 from http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_13.pdf.
U.S. Department of Health and Human Services. Organ Procurement and Transplantation Network (OPTN). (2010, November). Minimum procurement standards for an organ procurement organization (OPO). Retrieved November 19, 2010 from http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_2.pdf.
U.S. Department of Health and Human Services. Organ Procurement and Transplantation Network (OPTN). (2010, November). Organ procurement, distribution and alternative systems for organ distribution or allocation. Retrieved November 19, 2010 from http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_6.pdf.
ORIGINAL EFFECTIVE DATE: 4/1980
MOST RECENT REVIEW DATE: 10/20/2011
ID_BT
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
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